Ngaba kukho ingxaki egazini lakho? Masifunde nge-hemoglobinopathy ngokulula!

Ngaba uhlala uziva udiniwe kwaye udiniwe? Mhlawumbi ulusu lwakho lumhlophe kancinci? Okanye kukho umntu kusapho lwakho oye waba nesifo segazi? Ke ngoko into endizakukuxelela yona ibaluleke kakhulu kuwe. Namhlanje siza kuthetha ngesifo segazi esibizwa ngokuba yiHemoglobinopathy, esinegama elintsonkothileyo, kodwa elibonwa kubantu abaninzi. Ungakhathazeki, siza kuthetha ngale nto ngokulula.

Yintoni iHemoglobinopathy? Masifumanise ngokuchanekileyo!

Ngamafutshane, i-hemoglobinopathy liqela lezifo ezizuzwe njengelifa ezichaphazela iproteni egazini lethu ebizwa ngokuba yi-hemoglobin. Ngoku mhlawumbi uyazibuza ukuba yintoni le hemoglobin, akunjalo?

Cinga ngale ndlela: i-hemoglobin ifana neqhawe elincinci ngaphakathi kwiiseli zethu ezibomvu zegazi. Yiyo ethatha i-oksijini emiphungeni yethu xa siphefumla kwaye iyisasaze emizimbeni yethu yonke. Isebenza njengeteksi ye-oksijini. Ngoko ke ukuba le hemoglobin ayiveliswanga kakuhle, okanye ukuba ayiveliswanga ngokwaneleyo, imizimba yethu ayifumani i-oksijini eyaneleyo. Kulapho ke iingxaki ziqala khona.

Le meko ifunyanwa njengelifa, oko kuthetha ukuba ibangelwa kukutshintsha kwezakhi zofuzo esizifumana kubazali bethu. Izigidi zabantu kwihlabathi liphela zihlushwa yile meko. Enyanisweni, lolona hlobo luqhelekileyo lwesifo segazi esizuzwe njengelifa. Abaphandi bafumanise iintlobo ezingaphezu kwama-600 ze-hemoglobinopathy!

Xa imizimba yethu ivelisa i-hemoglobin engaqhelekanga, okanye ingabi nakwaneleyo, iimpawu ezifana neentlungu zomzimba, ukudinwa, kunye nomonakalo kumalungu ahlukeneyo zinokubakho. Yiyo loo nto kubaluleke kakhulu ukuyiqonda kwangethuba. Yiyo loo nto oogqirha bevavanya abantwana ukuba banayo i-hemoglobinopathy kwangoko nje emva kokuba bezelwe.

Into ebalulekileyo kukuba iHemoglobinopathy ayisosifo esinokunyangeka ngokupheleleyo. Nangona kunjalo, ngonyango olufanelekileyo, singakwazi ukulawula iimpawu nokuthintela iingxaki.

Ziziphi iintlobo ze-hemoglobinopathy ezikhoyo?

Njengoko besitshilo ngaphambili, kukho amakhulu eentlobo zezi. Ezi ntlobo zihlala zibizwa ngamagama esiNgesi. Ezi ntlobo zibhekisa kwiindidi ezahlukeneyo zeproteni ye-hemoglobin kunye nolandelelwano abaphandi abazifumene ngalo. Oku kunceda oogqirha baqonde umahluko ochanekileyo wemfuza obandakanyekayo.

Nazi ezinye zezona ntlobo zixhaphakileyo ze-hemoglobinopathy:

• Isifo seHemoglobin C: Kulapho ihemoglobin eqhelekileyo ithathelwa indawo luhlobo olubizwa ngokuba yihemoglobin C.
• Isifo seHemoglobin E: Apha, ihemoglobin eqhelekileyo ithathelwa indawo yihemoglobin E.
• Isifo seHemoglobin D: Apha, ihemoglobin eqhelekileyo ithathelwa indawo yihemoglobin D.
• Isifo seHemoglobin SC:Le meko yenzeka ukuba ufumana ilifa le-sickle cell gene enye kunye ne-hemoglobin C gene enye.
• Isifo se-Hemoglobin SD: Apha, i-gene enye ye-sickle cell kunye ne-gene enye ye-hemoglobin D zizuzwa njengelifa.
• Isifo seHemoglobin SE: Apha, i-gene enye ye-sickle cell kunye ne-gene enye ye-hemoglobin E zizuzwa njengelifa.
• Isifo seSickle Cell: Kule meko, iiseli zakho ezibomvu zegazi ziba nombala ofana nowesickle. Oku kuzithintela ukuba zingahambi lula emithanjeni yegazi kwaye kunokubangela ukuba zivaleke.
• IiThalassemia: Kule meko, umzimba wakho awuvelisi i-hemoglobin eyaneleyo.

Oku kusenokuvakala ngathi kuyinkimbinkimbi kancinci, kodwa ugqirha angakuchazela ngakumbi.

Zithini iimpawu zoku? Jonga ukuba nawe unazo ezi...

Iimpawu ze-hemoglobinopathy zingahluka ukusuka kumntu ukuya komnye, kodwa kukho ezinye iimpawu eziqhelekileyo:

• Izandla neenyawo ezibandayo
• Umchamo omnyama (ukuba mnyama)
• Ukudinwa rhoqo
• Uphuhliso olulibazisekileyo ebantwaneni
• Ukutyheli kolusu namehlo (i-Jaundice)
• Ulusu olukhanyayo
• Ubunzima bokuphefumla, ukuphefumla kancinci
• Ukulala ngaphezu kwesiqhelo
• Ukudumba kwamalungu

Iintsana ezine-hemoglobinopathy enzima zinokubonisa iimpawu xa zizalwa. Ngamanye amaxesha, iimpawu zivela ebuntwaneni. Kwabantu abadala, ezi mpawu zinokuba nzima ngakumbi ngexesha lokwanda kwesi sifo (njengengxaki ye-sickle cell).

Kutheni le nto ibangela le hemoglobinopathy?

Eyona nto iphambili kukuba kukho utshintsho lwezakhi zofuzo oluchaphazela i-hemoglobin yakho. Ungalufumana olu tshintsho lwezakhi zofuzo kumama wakho, utata wakho, okanye bobabini. Oku kuthetha ukuba le asiyonto esinokuyilawula, yinto evela kwilifa.

Ngubani onokuba nethuba elikhulu lokuphuhlisa oku? (Izinto ezibangela umngcipheko)

Abanye abantu banamathuba amaninzi okufumana olu hlobo lwe-hemoglobinopathy. Makhe sijonge ukuba bangoobani:

• Ukuba imvelaphi yakho ivela eAfrika, eMeditera, eMzantsi-mpuma Asia okanye eNtshona Asia. (Ezi meko zinokubonwa naseSri Lanka)
• Ukuba umama wakho, utata, okanye umntakwenu une-hemoglobinopathy.
• Ukuba uhlala kwindawo apho uvavanyo lwe-hemoglobinopathy olusandul’ ukuzalwa lungenziwa rhoqo.

Ukuba une-hemoglobinopathy, kukho ithuba lokuba nomntwana wakho abe nesi sifo. Kungoko ke, kubaluleke kakhulu ukufuna iingcebiso zonyango ngaphambi kokuba uqale usapho.

Zeziphi iingxaki ezinokubangela oku?

Ukuba i-hemoglobinopathy ayilawulwa kakuhle, iingxaki ezahlukeneyo zinokwenzeka. Ezinye zazo zezi:

• I-anemia: Ezinye iintlobo ze-hemoglobinopathy zinokubangela i-anemia, nto leyo enokubangela ukuncipha kweeseli ezibomvu zegazi. Oku kunokubangela ukudinwa nobuthathaka obuninzi.
• Usulelo oluqhelekileyo: I-Hemoglobinopathy inokukhokelela ekubeni inkqubo yomzimba yokuzikhusela ibe buthathaka , nto leyo eyonyusa umngcipheko wokugula nokosuleleka.
• Ukonakala kwamalungu omzimba: I-Hemoglobinopathy ibangela ukwehla kwamanqanaba eoksijini egazini. Xa igazi elinomoya-mpilo omninzi lingafikiswanga ngokufanelekileyo kangangexesha elithile, izicubu zethu kunye namalungu omzimba anokonakala.
• Iziqendu zentlungu: Kwezinye iintlobo ze-hemoglobinopathy, ezifana nesifo se-sickle cell, amahlwili egazi anokwakheka ngaphakathi kwemithambo yegazi aze ayivale. Oku kunokukhokelela kwiintlungu eziqatha kunye nokuncipha kokuhamba kwegazi.

Ungayixilonga njani ngokuchanekileyo le sifo? (Ukuxilongwa)

Oogqirha benza iimvavanyo ezininzi ukuze bafumanise ukuba ngaba une-hemoglobinopathy.

• Ubalo lwegazi olupheleleyo (CBC): Olu luvavanyo lokuqala lwegazi oluqhelekileyo. Lunokukuxelela ngeentlobo zeeseli egazini lakho, oko kukuthi, zingaphi kuhlobo ngalunye lweseli onalo.
• Uvavanyo lwemfuza: Olu vavanyo lunokuchonga kanye kanye ukuba loluphi utshintsho lwemfuza olubandakanyekayo.
• I-Hemoglobin electrophoresis: Olu vavanyo lwahlula iimolekyuli ze-hemoglobin kwisampuli yegazi lakho kwaye lukhangela izinto ezingaqhelekanga.
• Izifundo ze-iron: Olu vavanyo lungenziwa ukuze kufunyaniswe ukuba unayo na i-anemia yokunqongophala kwe-iron.
• Ukuhlolwa kweentsana ezisandul’ ukuzalwa: Kumazwe amaninzi afana neKhanada neMelika, iintsana ezisandul’ ukuzalwa ziyahlolwa i-hemoglobinopathy. Ezinye izibhedlele eSri Lanka nazo zinale ndawo.
• Uvavanyo lwangaphambi kokuzalwa: Olu vavanyo lungasetyenziselwa ukufumanisa ukuba umntwana ongekazalwa une-hemoglobinopathy ngexesha lokukhulelwa.

Zithini iindlela zonyango?

Kukho iindlela ezahlukeneyo zonyango lwe-hemoglobinopathy, kodwa ezi ndlela zonyango ziyahluka ngokuxhomekeke kuhlobo kunye nobunzima besifo.

• Utofelo-gazi: Ezinye iimeko, ezifana ne-thalassemia kunye nesifo se-sickle cell, zichaphazela ubungakanani be-hemoglobin egazini. Utofelo-gazi lunokufuneka ukuze kugcinwe amanqanaba e-hemoglobin aqhelekileyo.
• Iipilisi ze-folic acid: Ezi zinokunceda ukwandisa imveliso yeeseli ezibomvu zegazi.
• Unyango lwe-gene:Olu lunyango olutsha. Izazinzulu zithatha iiseli ezichaphazelekileyo emzimbeni wakho, zizilungise zisebenzisa iindlela ezikhethekileyo, zize ziphinde zizibuyisele emzimbeni wakho. Kufana nokubhala kwakhona imiyalelo kwiijini zakho.
• Unyango lwe-iron chelation: Ukuba une-iron eninzi emzimbeni wakho, olu nyango lunokususa i-iron engaphezulu. Eli yeza linikwa ngomlomo okanye njengenaliti.
• Unyango lweoksijini: Ugqirha wakho unokucebisa unyango lweoksijini kunye nezinye iindlela zonyango. Injongo yoku kukunyusa amanqanaba eoksijini egazini lakho nokunciphisa iintlungu.
• Ukufakelwa kweeseli zesiqu: Oku kuquka ukutshintsha iiseli ezibomvu zegazi ezingaqhelekanga ngeeseli ezibomvu zegazi eziphilileyo. Nangona kunjalo, oku akuqhelekanga kangako, njengoko kunzima ukufumana umntu ofanelekileyo onikelayo. Kukwanomngcipheko wesifo se-graft-versus-host (GvHD) .

Khumbula, kuxhomekeke kuhlobo lwe-hemoglobinopathy onayo, usenokungadingi naluphi na unyango konke konke. Ngoko ke kungcono ukuthetha nogqirha wakho ubuze ukuba yintoni ekulungeleyo.

Ndiza kuziva bhetele nini emva konyango?

Kuxhomekeke kakhulu kuhlobo lwe-hemoglobinopathy onayo kunye nobunzima bayo. Uninzi lwabantu luqala ukuziva lungcono kwiiveki okanye kwiinyanga ezimbalwa emva kokuqala unyango.

I-Hemoglobinopathy yimeko ehlala ixesha elide, ngoko ke kuya kufuneka uvavanywe rhoqo ngugqirha kwaye ubekwe esweni. Ugqirha wakho uya kukunceda ulawule iimpawu zakho.

Liyintoni ikamva labo baphila nesi sifo?

Ukuba unyango olufanelekileyo lunokuphucula ikamva lakho. Abantu abangaphezu kwama-90% abane-hemoglobinopathy baphila baze babe ngabantu abadala.

Nangona kunjalo, ukuba azinyangwa, iintlobo ezininzi ze-hemoglobinopathy zinokukhokelela ekufeni kwiminyaka embalwa yokuqala yobomi. Ke ngoko, ukuxilongwa kwangoko kunye nonyango yeyona ndlela ilungileyo yokuthintela iingxaki ezinkulu.

Ngaba oku akunakuthintelwa?

Ngelishwa, hayi. Ekubeni i-hemoglobinopathy yinto oyizuz’ ilifa, akukho ndlela yokuyithintela. Nangona kunjalo, singazilawula iimpawu ngonyango olufanelekileyo.

Ndingazinyamekela njani?

Ukuphila ne-hemoglobinopathy kunokuba nzima, kodwa ukuba uzinyamekela kakuhle, unganciphisa umngcipheko weentlungu kunye nezinye iziphumo ebezingalindelekanga.

Nazi ezinye iingcebiso ngokubanzi ezinokukunceda:

• Sela amanzi amaninzi. Zama ukusela ubuncinane iilitha ezi-2-3 zamanzi ngosuku.
• Yiba nomdla womzimba, kodwa ungawusebenzisi kakhulu. Mamela umzimba wakho. Ukuba uziva udiniwe, phumla.
• Yitya ukutya okutyebileyo kwi-iron, iivithamini, kunye neeminerali. Faka imifuno eluhlaza, iziqhamo, imifuno, inyama, kunye nentlanzi ekutyeni kwakho.
• Lawula uxinzelelo lwakho. Sebenzisa ukucamngca, i-yoga, okanye ezinye iindlela zokuzilolonga ozithandayo.
• Hlamba izandla zakho rhoqo. Oku kuyakunceda ekukhuselekeni kwiintsholongwane.

Ndifanele ndimbone nini ugqirha?

Ukuba uneempawu ezifana nokudinwa kakhulu, ubunzima bokuphefumla, okanye ulusu olukhanyayo, qiniseka ukuba ubona ugqirha kwaye umxelele ngako.

Ukuba sele ufunyenwe une-hemoglobinopathy, bonana nogqirha wakho ngokukhawuleza ukuba iimpawu zakho ziyabuya okanye ziya zisiba mandundu. Angalutshintsha unyango lwakho.

Yeyiphi imibuzo omele uyibuze ugqirha?

Ukuba wena okanye umntwana wakho une-hemoglobinopathy, kungcono ukubuza ugqirha wakho le mibuzo:

• Luhlobo luni lwe-hemoglobinopathy endinalo mna/umntwana wam?
• Luhlobo luni lonyango olucetyiswayo?
• Ngaba kuya kufuneka ndidibane nezinye iingcali ukuze ndifumane unyango?
• Ngaba kufuneka nditshintshe indlela enditya ngayo okanye indlela endizilolonga ngayo?
• Ingaba kunokwenzeka ukuba abantwana bam babe nale meko?
• Ngaba unalo olunye ulwazi malunga noku? (umz. iincwadi, iiwebhusayithi)

Kufuneka uye nini kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) ?

Ukuba unezinye zeempawu ezilandelayo, fowunela ku-911 (1990 eSri Lanka) okanye uye kwigumbi likaxakeka elikufutshane ngoko nangoko:

• Iintlungu zesifuba
• Isiyezi
• Umkhuhlane ongaphezulu kwama-degrees Fahrenheit ayi-103 (39.4 degrees Celsius)
• Intlungu ebuhlungu engapheziyo nangona usela amayeza
• Iimpawu zestroke, umzekelo, ukulahlekelwa yingqondo ngesiquphe, ubunzima bokuthetha.

I-hemoglobinopathy iyichaphazela njani i-A1C yam?

I-Hemoglobinopathy inokubangela ukuba iziphumo zakho zovavanyo lwe -A1C zingachaneki. Ngokuxhomekeke kuhlobo lwakho lwemfuza, ingabonisa ixabiso eliphantsi okanye eliphezulu kunelo unalo ngokwenene.

Ukuba une-hemoglobinopathy, ugqirha wakho unokucebisa uvavanyo olunokunceda ukunciphisa le ngxaki. Le A1C ibaluleke kakhulu kubantu abanesifo seswekile, ngoko ungalibali ukuthetha nogqirha wakho ngale nto.

Okokugqibela, ezona zinto zibalulekileyo ekufuneka uzikhumbule!

Kuqhelekile ukuba uzive usoyika kwaye uxhalabile xa ufumanisa ukuba wena okanye umntwana wakho unesifo segazi esihlala ixesha elide. Imibuzo efana nethi "I-hemoglobinopathy iza kuyichaphazela njani ubomi bam?", "Ngaba ndiza kuhlala ndisezintlungwini?", "Esi sifo siza kumchaphazela njani umntwana wam?" inokuvela engqondweni yakho.

Kodwa iindaba ezimnandi zezokuba ngonyango olufanelekileyo kunye nokulawula iimpawu, ungaphila ubomi obusempilweni kangangoko unako.Eyona nto ingcono onokuyenza kukuthetha nogqirha wakho. Musa ukulibazisa. Ukuxilongwa kwangoko kunye nonyango zezona zinto zibalulekileyo ebomini obude nobunempilo.

Ungakhathazeki, awuwedwa. Oogqirha, abahlengikazi, kunye nosapho lwakho baya kukunceda kolu hambo. Yiba nesibindi!

I- Hemoglobinopathy, izifo zegazi, izifo ezizuzwa njengelifa, i-hemoglobin, i-anemia, isifo se-sickle cell, i-thalassemia